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AJR 2004; 183:1843-1844
© American Roentgen Ray Society

MRI of Angiolipoma of the Breast in Turner's Syndrome

J. Keith Killian, Maria Merino, Carolyn Bondy, Vladimir Bakalov and Catherine Chow

National Institutes of Health Bethesda, MD 20892

A 29-year-old woman with cytogenetically confirmed Turner's syndrome (45,X) underwent chest MRI for evaluation of cardiovascular anomalies. In addition to a bicuspid aortic valve and a mild to moderate coarctation of the aorta, a 6-mm ovoid focus of enhancement in the right breast was noted. On follow-up dedicated breast MRI, the lesion appeared isointense relative to the surrounding breast parenchyma (not shown) and displayed rapid washout of contrast material (Fig. 6A). Mammography was not performed because the likelihood of visualizing this lesion in a dense breast was low. Sonography failed to reveal the lesion, and repeat MRI was performed 3 months after cessation of the patient's hormonal replacement therapy. Because of the persistent presence of theFig. 6B suspicious lesion on MRI, MRI-guided localization and an excisional biopsy were performed. Pathologic examination revealed an 8 x 7 mm lesion diagnosed as an angiolipoma surrounded by a highly fibrotic breast (Figs. 6C and 6D).



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Fig. 6A. 29-year-old woman with Turner's syndrome and angiolipoma of breast. Contrast-enhanced sagittal fat-suppressed fast spoiled gradient–recalled echo image shows well-defined, ovoid, brightly enhancing nodule.

 


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Fig. 6B. 29-year-old woman with Turner's syndrome and angiolipoma of breast. Time–intensity curve from dynamic MRI examination shows rapid uptake and washout of contrast material. On x-axis, time is plotted in 30-sec intervals; on y-axis, enhancement is calculated with formula: signal intensity after contrast injection / signal intensity before contrast injection.

 


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Fig. 6C. 29-year-old woman with Turner's syndrome and angiolipoma of breast. Photomicrographs of histopathologic specimen of breast angiolipoma were obtained at 100x (C) and 400x (D). (H and E)

 


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Fig. 6D. 29-year-old woman with Turner's syndrome and angiolipoma of breast. Photomicrographs of histopathologic specimen of breast angiolipoma were obtained at 100x (C) and 400x (D). (H and E)

 

Although adipose tissue is an important component of normal breast tissue, breast lipomas are unusual, and the angiolipoma variant is rare. The ratio of the two mesenchymal elements of angiolipomas—mature white fat and capillary-sized vessels—varies. The vessels often appear as lobulated collections at the tumor periphery extending as streaks toward the fatty center. Vessels may be plugged with eosinophilic fibrin microthrombi. The lobular collections of vessels show absent communication among individual vessels, a feature that may be used to distinguish cellular angiolipomas from well-differentiated angiosarcomas. These features and the lack of necrosis, mitoses, cellular atypia, or piling up of endothelial cells help separate angiolipomas from malignant vascular tumors [1, 2]

Angiolipoma typically is found in young adults, predominately in men, and frequently occurs as multiple tumors. Angiolipoma of the breast, unlike angiolipoma at other sites, usually is painless. All karyotyped angiolipomas except one have been reported to show normal karyotype [3, 4]. The significance of Turner's syndrome in the pathogenicity of our patient is not known. Turner's syndrome occurs in approximately one of every 2,500 live female births, with short stature and hypogonadism being the most common clinical findings [5]. Anatomic features such as lymphedema, skeletal abnormalities, and congenital heart and kidney defects are found in approximately 50% of affected individuals [5, 6], including this patient, who had a bicuspid aortic valve, coarctation of the aorta, and abnormal right kidney associated with marked lymphedema at birth and prominent neck webbing.

Regarding tumorigenesis, the presence of Y chromosome elements is associated with increased risk for gonadoblastoma in patients with Turner's syndrome [7], and the risk for neuroblastoma may be increased among patients with 45,X karyotype [8]. The incidence of noncancerous hamartomatous growths, such as pigmented nevi, gastrointestinal telangiectatic lesions, and capillary hemangiomas, also is curiously increased in patients with Turner's syndrome [3, 4]. Our patient has been on estrogen–progestin treatment for ovarian failure since the age of 13 years and had received recombinant human growth hormone for 5 years during childhood. Growth hormone may stimulate lipoma growth [9, 10], so this treatment could have contributed to the development of an unusual breast lesion in this patient.


References
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